The clinical sequelae of intravascular hemolysis and extracellular plasma hemoglobin: a novel mechanism of human disease

RP Rother, L Bell, P Hillmen, MT Gladwin - Jama, 2005 - jamanetwork.com
ContextThe efficient sequestration of hemoglobin by the red blood cell membrane and the
presence of multiple hemoglobin clearance mechanisms suggest a critical need to prevent …

The red blood cell—inflammation vicious circle in sickle cell disease

E Nader, M Romana, P Connes - Frontiers in immunology, 2020 - frontiersin.org
Sickle cell disease (SCD) is a genetic disease caused by a single mutation in the β-globin
gene, leading to the production of an abnormal hemoglobin called hemoglobin S (HbS) …

Lactate dehydrogenase as a biomarker of hemolysis-associated nitric oxide resistance, priapism, leg ulceration, pulmonary hypertension, and death in patients with …

GJ Kato, V McGowan, RF Machado, JA Little, J Taylor… - Blood, 2006 - ashpublications.org
Pulmonary hypertension is prevalent in adult patients with sickle cell disease and is strongly
associated with early mortality and markers of hemolysis, in particular, serum lactate …

Pulmonary complications of sickle cell disease

MT Gladwin, E Vichinsky - New England journal of medicine, 2008 - Mass Medical Soc
This review presents evidence for two overlapping yet distinctive clinical types of sickle cell
disease. The basis of one is the vaso-occlusive crisis; the other is the consequence of …

Pathophysiology and treatment of stroke in sickle-cell disease: present and future

JA Switzer, DC Hess, FT Nichols, RJ Adams - The Lancet Neurology, 2006 - thelancet.com
Sickle-cell anaemia is the most common cause of stroke in children, and stroke is one of the
most devastating complications of sickle-cell disease. Overt strokes are typically due to large …

Hemolysis-associated endothelial dysfunction mediated by accelerated NO inactivation by decompartmentalized oxyhemoglobin

PC Minneci, KJ Deans, H Zhi, PST Yuen… - The Journal of …, 2005 - Am Soc Clin Investig
During intravascular hemolysis in human disease, vasomotor tone and organ perfusion may
be impaired by the increased reactivity of cell-free plasma hemoglobin (Hb) with NO. We …

Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability

LL Hsu, HC Champion, SA Campbell-Lee… - Blood, 2007 - ashpublications.org
Pulmonary hypertension is a highly prevalent complication of sickle cell disease and is a
strong risk factor for early mortality. However, the pathophysiologic mechanisms leading to …

Sickle cell disease vasculopathy: a state of nitric oxide resistance

KC Wood, LL Hsu, MT Gladwin - Free radical biology and medicine, 2008 - Elsevier
Sickle cell disease (SCD) is a hereditary hemoglobinopathy characterized by microvascular
vaso-occlusion with erythrocytes containing polymerized sickle (S) hemoglobin, erythrocyte …

Erythrocyte glutamine depletion, altered redox environment, and pulmonary hypertension in sickle cell disease

CR Morris, JH Suh, W Hagar, S Larkin… - Blood, The Journal …, 2008 - ashpublications.org
Erythrocyte glutathione depletion has been linked to hemolysis and oxidative stress.
Glutamine plays an additional antioxidant role through preservation of intracellular …

Levels of soluble endothelium‐derived adhesion molecules in patients with sickle cell disease are associated with pulmonary hypertension, organ dysfunction, and …

GJ Kato, S Martyr, WC Blackwelder… - British journal of …, 2005 - Wiley Online Library
Endothelial cell adhesion molecules orchestrate the recruitment and binding of inflammatory
cells to vascular endothelium. With endothelial dysfunction and vascular injury, the levels of …